Linked Life Data

14763337

Source:http://linkedlifedata.com/resource/pubmed/id/14763337

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2004-2-6
pubmed:abstractText
Many circumstances lead to discover a neutropenia in paediatric practice. In most of the cases, it is an acquired, transient neutropenia, related to a viral or a bacterial infection, a malignant haemopathy, or an acquired auto-immune neutropenia, also called benign chronic neutropenia. Constitutional disorder with neutropenia is more exceptional. Many complex genetic diseases include a neutropenia, among which several immunologic disorders that could be easily diagnosed by immunological tests. Other complex genetic diseases include Shwachman-Diamond syndrome, associating an external pancreatic insufficiency with bone and skin abnormalities; Glycogen storage disease type Ib, with metabolic disorder. Primary constitutional neutropenias are limited to very few entities. Kostmann's disease is a permanent isolated neutropenia, usually associated with a bone marrow granulopoeisis blockage; cyclic neutropenia is characterised by recurrent oscillations (every 21 days) of neutrophil count. Elastase 2 gene mutations have been observed in both diseases. Treatment and prevention of severe infections are a major concern in the management of chronic neutropenia and could be achieved by prophylactic antibiotics (like sulfamethoxazole-trimethoprime) and also G-CSF, which is regularly effective, but could facilitate various side-effects. Constitutional neutropenias, especially Kostmann's disease and Shwachman-Diamond syndrome, are associated with an increased leukemic risk.
pubmed:language
fre
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
0929-693X
pubmed:author
pubmed:issnType
Print
pubmed:volume
10 Suppl 4
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
521s-523s
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
2003
pubmed:articleTitle
[Evaluation of neutropenia in children].
pubmed:affiliation
Service d'hématologie et oncologie pédiatrique, hôpital Trousseau AP-HP, 26, avenue du Dr Netter, 75012 Paris, France. jean.donadieu@trs.ap-hop-paris.fr
pubmed:publicationType
Journal Article, English Abstract